Primary Biliary Cirrhosis
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PRIMARY BILIARY CIRRHOSIS 1.
CHIEF COMPLAINT •Primary biliary cirrhosis
2.
HPI
3.
ROS
4.
SYMPTOMS •Fatigue •Depression •Obsessive-Compulsive Behavior •Daytime somnolence •Pruritus •Right upper quadrant discomfort
5.
HISTORY •FAMILY HISTORY o Primary biliary cirrhosis
6. 7.
•SOCIAL HISTORY •PAST MEDICAL HISTORY •SURGICAL HISTORY •CHRONIC CONDITIONS ALLERGIES PHYSICAL EXAMINATION •Excoriations of the skin •Xanthelasmata •Hepatomegaly •Hyperpigmentation •Splenomegaly •Jaundice •Xanthelasmata •Xerophthalmia •Xerostomia •Kayser-Fleischer rings •Spider nevi •Palmar erythema •Ascites •Temporal and proximal muscle wasting
•Peripheral edema 8.
SPECIFIC DATA LIKE GRADING ETC •Stage 1 (portal stage of Ludwig): Portal inflammation, bile duct abnormalities, or both are present. •Stage 2 (periportal stage): Periportal fibrosis is present, with or without periportal inflammation or prominent enlargement of the portal tracts with seemingly intact, newly formed limiting plates. •Stage 3 (septal stage): Septal fibrosis with active inflammatory, passive paucicellular septa or both are present. •Stage 4 (cirrhosis): Nodules with various degrees of inflammation are present.
9.
TESTS TO BE ORDERED •CBP •Serum aminotransferases •Serum alanine aminotransferase •Serum aspartate aminotransferase (AST) •Serum alkaline phosphatase (ALP) •Serum g-glutamyl transpeptidase (GGTP) •Immunoglobulin levels (mainly immunoglobulin M [IgM]) •Lipid profile •ESR •Serum bilirubin •Prothrombin time •Serum albumin •Antinuclear antibodies (ANAs) •Abdominal ultrasonography •CT scan •MRI •Percutaneous or laparoscopic liver biopsy •Upper endoscopy
10. ASSESSMENT /PLAN Surgical Care •Liver transplantation appears to be the only life-saving procedure. Activity
•Increased activity is recommended, especially in postmenopausal women, to prevent osteoporosis. 11. EDUCATION 12. MEDICATION •Ursodeoxycholic acid (UDCA) •Methotrexate •Cyclosporine •Colchicine •Cholestyramine •Colestipol •Rifampin •Dronabinol •Plasmapheresis •Ursodiol •Prednisolone •Hydroxyzine hydrochloride 13. FOLLOW-UP
CHIEF COMPLAINT •Primary biliary cirrhosis
2.
HPI
3.
ROS
4.
SYMPTOMS •Fatigue •Depression •Obsessive-Compulsive Behavior •Daytime somnolence •Pruritus •Right upper quadrant discomfort
5.
HISTORY •FAMILY HISTORY o Primary biliary cirrhosis
6. 7.
•SOCIAL HISTORY •PAST MEDICAL HISTORY •SURGICAL HISTORY •CHRONIC CONDITIONS ALLERGIES PHYSICAL EXAMINATION •Excoriations of the skin •Xanthelasmata •Hepatomegaly •Hyperpigmentation •Splenomegaly •Jaundice •Xanthelasmata •Xerophthalmia •Xerostomia •Kayser-Fleischer rings •Spider nevi •Palmar erythema •Ascites •Temporal and proximal muscle wasting
•Peripheral edema 8.
SPECIFIC DATA LIKE GRADING ETC •Stage 1 (portal stage of Ludwig): Portal inflammation, bile duct abnormalities, or both are present. •Stage 2 (periportal stage): Periportal fibrosis is present, with or without periportal inflammation or prominent enlargement of the portal tracts with seemingly intact, newly formed limiting plates. •Stage 3 (septal stage): Septal fibrosis with active inflammatory, passive paucicellular septa or both are present. •Stage 4 (cirrhosis): Nodules with various degrees of inflammation are present.
9.
TESTS TO BE ORDERED •CBP •Serum aminotransferases •Serum alanine aminotransferase •Serum aspartate aminotransferase (AST) •Serum alkaline phosphatase (ALP) •Serum g-glutamyl transpeptidase (GGTP) •Immunoglobulin levels (mainly immunoglobulin M [IgM]) •Lipid profile •ESR •Serum bilirubin •Prothrombin time •Serum albumin •Antinuclear antibodies (ANAs) •Abdominal ultrasonography •CT scan •MRI •Percutaneous or laparoscopic liver biopsy •Upper endoscopy
10. ASSESSMENT /PLAN Surgical Care •Liver transplantation appears to be the only life-saving procedure. Activity
•Increased activity is recommended, especially in postmenopausal women, to prevent osteoporosis. 11. EDUCATION 12. MEDICATION •Ursodeoxycholic acid (UDCA) •Methotrexate •Cyclosporine •Colchicine •Cholestyramine •Colestipol •Rifampin •Dronabinol •Plasmapheresis •Ursodiol •Prednisolone •Hydroxyzine hydrochloride 13. FOLLOW-UP
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